Introduction: Autoimmune hepatitis (AIH) is a rare association of systemic sclerosis. Even rarer is the association of AIH with localized scleroderma, with only two reported cases in literature up to date. We hereby report a case of AIH associated with localized scleroderma. Case description: A 39-year-old female with localized scleroderma presented to the hospital with periumbilical pain, non-bloody emesis, and jaundice. Physical exam showed normal vitals with scleral icterus, thickened skin plaques affecting all extremities, and a normal abdominal exam. Laboratory findings were significant for a Hg of 10.9 gm/dL with an MCV of 74.5 and platelets of 143 k/dL. LFTs showed a total protein of 7.3 g/dL with an albumin 3.7 of g/dL, total bilirubin of 9.5 mg/dL, AST of 1288 U/L, ALT of 775 U/L, ALP of 184 U/L, GGT of 196 U/L, LDH of 760 U/L, and an INR of 1.25. Work-up of chronic liver disease, including serologies for hepatitis A, B and C, EBV, CMV, HSV, and ferritin iron panel were unremarkable. Furthermore, autoimmune serologies, including antibodies to nuclear, mitochondrial, smooth muscle, liver cytosol, parietal cells, and liver-kidney microsome type 1 were all negative. Imaging of abdomen and pelvis with US Doppler and CT scan were unremarkable. Liver biopsy showed plasma cell infiltration, rosette formation, and interface hepatitis histology consistent with autoimmune hepatitis. The patient was started on steroids and azathioprine, with complete normalization of LFTs. Discussion: Localized scleroderma, a rare autoimmune disorder with female predominance, is associated with other autoimmune conditions. While the pathological mechanisms remain unclear, activation of the cellular and humoral immune responses may play a part. Association of AIH and scleroderma has been limited to two prior case reports. We suggest that a liver biopsy be considered in patients with localized scleroderma, with persistent elevation of liver enzymes in the setting of negative autoimmune serologies, to make a diagnosis of AIH.